Anatomical distribution, the incidence of malignancy and diagnostic workup in the pathological lesions of the clavicle: a review of 410 cases.

BACKGROUND: The clavicle poses a diagnostic dilemma of the pathological lesions due to the wide range of pathologies seen at this site. This study aimed to identify and stratify various pathologies seen in the clavicle and to guide ways of investigation for diagnosis based on age, site and investigation findings. MATERIALS AND METHODS: Four hundred and ten cases with clavicle lesions were identified in our database. Data were collected about the patient's medical history, previous investigation, inflammatory markers radiological investigations and biopsy. All patients were worked up and managed after discussion in a multidisciplinary team meeting (MDT). RESULTS: Non-malignant lesions accounted for 79% of cases. Infection was the most common diagnosis (39%) and the commonest diagnosis in those less than 20 years of age. 73% of the lesions were found at the medial end of the clavicle. Malignant tumours were 21%, while primary benign bone tumours accounted for only 14%. 50% of the malignant lesions were due to metastatic disease. The risk of malignancy increases with advancing age. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were not sensitive as a diagnostic tool in cases of osteomyelitis confirmed by histology. Magnetic resonance imaging (MRI) was noted to have high sensitivity and specificity for identifying the nature of a lesion and diagnosis. CONCLUSION: We have identified age as a positive predictor of a malignant cause in pathological lesions of the clavicle. MRI should be considered in all these cases. CRP and ESR have poor predictive values in diagnosing infection in the clavicle. Patients presenting with clavicle lesions should be discussed in a specialist MDT and undergo a systemic diagnostic workup, still in some cases, diagnosis can be speculated based on the patient's age, location of the lesion within the clavicle and the features seen on the MRI scan. LEVEL OF EVIDENCE: IV.

Denosumab in patients with giant-cell tumour of bone: a multicentre, open-label, phase 2 study.

BACKGROUND: Giant-cell tumour of bone (GCTB) is a rare, locally aggressive osteoclastogenic stromal tumour of the bone. This phase 2 study aimed to assess the safety and activity of denosumab in patients with surgically salvageable or unsalvageable GCTB. METHODS: In this multicentre, open-label, phase 2 study done at 30 sites in 12 countries we enrolled adults and skeletally mature adolescents (aged ≥12 years) weighing at least 45 kg with histologically confirmed and radiographically measurable GCTB, Karnofsky performance status 50% or higher (Eastern Cooperative Oncology Group status 0, 1, or 2), and measurable active disease within 1 year of study enrolment. Patients had surgically unsalvageable GCTB (cohort 1), had surgically salvageable GCTB with planned surgery expected to result in severe morbidity (cohort 2), or were enrolled from a previous study of denosumab for GCTB (cohort 3). Patients received 120 mg subcutaneous denosumab once every 4 weeks during the treatment phase, with loading doses (120 mg subcutaneously) administered on study days 8 and 15 to patients in cohorts 1 and 2 (patients in cohort 3 did not receive loading doses). The primary endpoint was safety in terms of the type, frequency, and severity of adverse events; secondary endpoints included time to disease progression from cohort 1 and the proportion of patients without surgery at month 6 for cohort 2. The safety analysis set included all enrolled patients who received at least one dose of denosumab. This study is registered with ClinicalTrials.gov, number NCT00680992, and has been completed. FINDINGS: Between Sept 9, 2008, and Feb 25, 2016, 532 patients were enrolled: 267 in cohort 1, 253 in cohort 2, and 12 in cohort 3. At data cutoff on Feb 24, 2017, median follow-up was 58·1 months (IQR 34·0-74·4) in the overall patient population, and 65·8 months (40·9-82·4) in cohort 1, 53·4 months (28·2-64·1) in cohort 2, and 76·4 months (61·2-76·5) in cohort 3. During the treatment phase, the most common grade 3 or worse adverse events were hypophosphataemia (24 [5%] of 526 patients), osteonecrosis of the jaw (17 [3%], pain in extremity (12 [2%]), and anaemia (11 [2%]). Serious adverse events were reported in 138 (26%) of 526 patients; the most common were osteonecrosis of the jaw (17 [3%]), anaemia (6 [1%]), bone giant cell tumour (6 [1%]), and back pain (5 [1%]). 28 (5%) patients had positively adjudicated osteonecrosis of the jaw, four (1%) had atypical femur fracture, and four (1%) had hypercalcaemia occurring 30 days after denosumab discontinuation. There were four cases (1%) of sarcomatous transformation, consistent with historical data. Ten (2%) treatment-emergent deaths occurred (two of which were considered treatment-related; bone sarcoma in cohort 2 and sarcoma in cohort 1). Median time to progression or recurrence for patients in cohort 1 during the first treatment phase was not reached (28 [11%] of 262 patients had progression or recurrence). 227 (92%; 95% CI 87-95) of 248 patients who received at least one dose of denosumab in cohort 2 had no surgery in the first 6 months of the study. INTERPRETATION: The types and frequencies of adverse events were consistent with the known safety profile of denosumab, which showed long-term disease control for patients with GCTB with unresectable and resectable tumours. Our results suggest that the overall risk to benefit ratio for denosumab treatment in patients with GCTB remains favourable. FUNDING: Amgen.

Surgical treatment for pelvic Ewing sarcoma: What is a safe and functional acetabular reconstruction when combined with modern multidisciplinary treatments?

PURPOSE: The purpose of this study was to clarify which local treatment is oncologically and functionally effective in pelvic Ewing sarcoma (ES). METHODS: A consecutive series of patients who underwent pelvic resections and acetabular reconstructions after chemotherapy between 1986 and 2016 at a supra-regional center were evaluated. RESULTS: The cohort consisted of 35 patients. The 5-year overall survival (OS) and local recurrence-free survival (LRFS) was 61% and 72%, respectively. Preoperative radiotherapy (RT) and surgery provided an excellent/good histological response in 92% and achieved significantly better OS (5 years, 64%) and LRFS (5 years, 100%) than surgery alone or surgery with postoperative RT. The Musculoskeletal Tumor Society functional scores were significantly better in patients with hip transposition than those with structural reconstructions (74% vs 57%; P = .031) using custom-made prostheses, irradiated autografts, and ice-cream cone prostheses. These scores were significantly lower if patients had deep infection (P = .035), which was the most common complication (28%) in structural reconstructions but did not occur in hip transposition even when performed after preoperative RT. CONCLUSION: Acetabular reconstruction with hip transposition resulted in no deep infection and superior function in patients with pelvic ES even when combined with preoperative RT, which improved tumor necrosis and rate of local control and survival.

Hypotensive Epidural Anesthesia Reduces Blood Loss in Pelvic and Sacral Bone Tumor Resections.

BACKGROUND: Resection of pelvic and sacral tumors can cause severe blood loss, complications, and even postoperative death. Hypotensive epidural anesthesia has been used to mitigate blood loss after elective arthroplasty, but to our knowledge, it has not been studied as an approach that might make resection of pelvic and sacral tumors safer. QUESTIONS/PURPOSES: The purposes of this study were (1) to compare the blood loss and blood product use for patients undergoing pelvic and sacral tumor surgery under standard anesthesia or hypotensive epidural anesthesia; (2) to assess the frequency of end-organ damage with the two techniques; and (3) to compare 90-day mortality between the two techniques. METHODS: Between 2000 and 2014, 285 major pelvic and sacral resections were performed at one center. A total of 174 (61%) had complete data sets for analysis of blood loss, transfusion use, complications, and mortality at 90 days. Of those, 102 (59%) underwent hypotensive epidural anesthesia, whereas the remainder received standard anesthetic care. The anesthetic approach was determined by the anesthetists in charge of the case with hypotensive epidural anesthesia exclusively performed by one of two subspecialty trained anesthetists as their routine for major pelvic or sacral surgery. The groups were comparable in terms of potential confounding variables such as age, gender, tumor volume, and operation performed. Hypotensive epidural anesthesia was defined as a technique using an extensive epidural block up to T2-3 dermatome, peripherally administered low-concentration intravenous adrenaline infusion, and using unimpeded spontaneous respiration to achieve controlled hypotension, precise rate control of the heart, and enhanced velocity of venous return, all aggregated thus to minimize blood loss during pelvic surgery while preserving vital perfusion. The groups were assessed for perioperative blood loss calculated from pre- and postsurgery hemoglobin and transfusion use as well as postoperative complications, morbidity, and mortality at 90 days. RESULTS: There was less mean blood loss in the hypotensive epidural anesthesia group (1457 mL, SD 1721, 95% confidence interval [CI], 1114-1801 versus 2421 mL, SD 2297, 95% CI, 1877-2965; p = 0.003). Patients in the hypotensive epidural anesthesia group on average received fewer packed red cell transfusions (2.7 units, SD 2.9, 95% CI, 2.1-3.2 versus 3.9 units, SD 4.4, 95% CI, 2.9-5.0; p = 0.03). There were no differences in the proportions of patients experiencing end-organ injury (7%, n = seven of 102 versus 6%, n = four of 72; p = 0.72). With the numbers available, there was no difference in 90-day mortality rate between groups (1.9%, n = two of 102 versus 1.3%, n = one of 72; p = 0.77). CONCLUSIONS: We found that hypotensive epidural anesthesia resulted in less blood loss, fewer transfusions, and no apparent increase in serious complications in pelvic and sacral tumor surgery performed in the setting of a high-volume tertiary sarcoma referral hospital. We recommend that further collaborative studies be undertaken to confirm our results with hypotensive epidural anesthesia in surgery for pelvic tumors. LEVEL OF EVIDENCE: Level III, therapeutic study.

Proximal Tibia Reconstruction After Bone Tumor Resection: Are Survivorship and Outcomes of Endoprosthetic Replacement and Osteoarticular Allograft Similar?

BACKGROUND: The proximal tibia is one of the most challenging anatomic sites for extremity reconstructions after bone tumor resection. Because bone tumors are rare and large case series of reconstructions of the proximal tibia are lacking, we undertook this study to compare two major reconstructive approaches at two large sarcoma centers. QUESTIONS/PURPOSES: The purpose of this study was to compare groups of patients treated with endoprosthetic replacement or osteoarticular allograft reconstruction for proximal tibia bone tumors in terms of (1) limb salvage reconstruction failures and risk of amputation of the limb; (2) causes of failure; and (3) functional results. METHODS: Between 1990 and 2012, two oncologic centers treated 385 patients with proximal tibial resections and reconstruction. During that time, the general indications for those types of reconstruction were proximal tibia malignant tumors or bone destruction with articular surface damage or collapse. Patients who matched the inclusion criteria (age between 15 and 60 years old, diagnosis of a primary bone tumor of the proximal tibia treated with limb salvage surgery and reconstructed with endoprosthetic replacement or osteoarticular allograft) were included for analysis (n = 149). In those groups (endoprosthetic or allograft), of the patients not known to have reached an endpoint (death, reconstructive failure, or limb loss) before 2 years, 85% (88 of 104) and 100% (45 of 45) were available for followup at a minimum of 2 years. A total of 88 patients were included in the endoprosthetic group and 45 patients in the osteoarticular allograft group. Followup was at a mean of 9.5 (SD 6.72) years (range, 2-24 years) for patients with endoprosthetic reconstructions, and 7.4 (SD 5.94) years for patients treated with allografts (range, 2-21 years). The following variables were compared: limb salvage reconstruction failure rates, risk of limb amputation, type of failures according to the Henderson et al. classification, and functional results assessed by the Musculoskeletal Tumor Society system. RESULTS: With the numbers available, after competitive risk analysis, the probability of failure for endoprosthetic replacement of the proximal tibia was 18% (95% confidence interval [CI], 10.75-27.46) at 5 years and 44% (95% CI, 31.67-55.62) at 10 years and for osteoarticular allograft reconstruction was 27% (95% CI, 14.73-40.16) at 5 years and 32% (95% CI, 18.65-46.18) at 10 years. There were no differences in terms of risk of failures at 5 years (p = 0.26) or 10 years (p = 0.20) between the two groups. Fifty-one of 88 patients (58%) with proximal tibia endoprostheses developed a reconstruction failure with mechanical causes being the most prevalent (32 of 51 patients [63%]). A total of 19 of 45 osteoarticular allograft reconstructions failed (42%) and nine of 19 (47%) of them were caused by early infection. Ten-year risk of amputation after failure for endoprosthetic reconstruction was 10% (95% CI, 5.13-18.12) and 11% (95% CI, 4.01-22.28) for osteoarticular allograft with no difference between the groups (p = 0.91). With the numbers available, there were no differences between the groups in terms of the mean Musculoskeletal Tumor Society score (26.58, SD 2.99, range, 19-30 versus 27.52, SD 1.91, range, 22-30; p = 0.13; 95% CI, -2,3 to 0.32). Mean extension lag was more severe in the endoprosthetic group than the osteoarticular allograft group: 13.56° (SD 18.73; range, 0°-80°) versus 2.41° (SD 5.76; range, 0°-30°; p < 0.001; 95% CI, 5.8-16.4). CONCLUSIONS: Reconstruction of the proximal tibia with either endoprosthetic replacement or osteoarticular allograft appears to offer similar reconstruction failures rates. The primary cause of failure for allograft was infection and for endoprosthesis was mechanical complications. We believe that the treating surgeon should have both options available for treatment of patients with malignant or aggressive tumors of the proximal tibia. (S)he might consider an allograft in a younger patient to achieve better extensor mechanism function, whereas in an older patient or one with a poorer prognosis where return to function and ambulation quickly is desired, an endoprosthesis may be advantageous. LEVEL OF EVIDENCE: Level III, therapeutic study.

Gene expression profiling identifies distinct molecular subgroups of leiomyosarcoma with clinical relevance.

BACKGROUND: Soft tissue sarcomas are heterogeneous and a major complication in their management is that the existing classification scheme is not definitive and is still evolving. Leiomyosarcomas, a major histologic category of soft tissue sarcomas, are malignant tumours displaying smooth muscle differentiation. Although defined as a single group, they exhibit a wide range of clinical behaviour. We aimed to carry out molecular classification to identify new molecular subgroups with clinical relevance. METHODS: We used gene expression profiling on 20 extra-uterine leiomyosarcomas and cross-study analyses for molecular classification of leiomyosarcomas. Clinical significance of the subgroupings was investigated. RESULTS: We have identified two distinct molecular subgroups of leiomyosarcomas. One group was characterised by high expression of 26 genes that included many genes from the sub-classification gene cluster proposed by Nielsen et al. These sub-classification genes include genes that have importance structurally, as well as in cell signalling. Notably, we found a statistically significant association of the subgroupings with tumour grade. Further refinement led to a group of 15 genes that could recapitulate the tumour subgroupings in our data set and in a second independent sarcoma set. Remarkably, cross-study analyses suggested that these molecular subgroups could be found in four independent data sets, providing strong support for their existence. CONCLUSIONS: Our study strongly supported the existence of distinct leiomyosarcoma molecular subgroups, which have clinical association with tumour grade. Our findings will aid in advancing the classification of leiomyosarcomas and lead to more individualised and better management of the disease.

Surgical downstaging in an open-label phase II trial of denosumab in patients with giant cell tumor of bone.

BACKGROUND: Surgical resection with curative intent for giant cell tumor of bone (GCTB) may be associated with severe morbidity. This interim analysis evaluated reduction in surgical invasiveness after denosumab treatment in patients with resectable GCTB. METHODS: Patients with primary or recurrent GCTB, for whom the initially planned surgery was associated with functional compromise or morbidity, received denosumab 120 mg subcutaneously every 4 weeks (additional doses on days 8 and 15 of the first cycle). Planned and actual GCTB-related surgical procedures before and after denosumab treatment were reported. Patients were followed for surgical outcome, adverse events, and recurrence following resection. RESULTS: Overall, 222 patients were evaluable for surgical downstaging (54 % were women; median age 34 years). Lesions (67 % primary and 33 % recurrent) were located in the axial (15 %) and appendicular skeleton (85 %). At the data cutoff date, most patients had not yet undergone surgery (n = 106; 48 %) or had a less morbid procedure (n = 84; 38 %) than originally planned. Median (interquartile range) time on denosumab was 19.5 (12.4-28.6) months for the 106 patients who had not undergone surgery and were continuing on monthly denosumab. Native joint preservation was 96 % (n = 24/25) for patients with planned joint/prosthesis replacement and 86 % (n = 30/35) for patients with planned joint resection/fusion. Of the 116 patients who had surgery (median postsurgical follow-up 13.0 [8.5-17.9] months), local recurrence occurred in 17 (15 %) patients. CONCLUSION: For patients with resectable GCTB, neoadjuvant denosumab therapy resulted in beneficial surgical downstaging, including either no surgery or a less morbid surgical procedure.

Management and prognostic significance of pathological fractures through chondrosarcoma of the femur.

PURPOSE: The aim of the study was to report overall survival, local recurrence and development of metastasis in a group of patients with femur chondrosarcoma that presented with or without a pathological fracture. METHODS: A retrospective review was performed, and 182 patients (39 pathological fractures) that had been treated by oncologic surgery for femoral chondrosarcoma were included. The mean age of the series was 50 years (range, eight to 90) and 114 patients were male (63%). Mean follow-up was 113 months (range, three to 216). Cancer-specific overall survival, development of metastasis and local recurrence were analysed, grouping patients by grade (grade I / grade 2-3 / dedifferentiated). RESULTS: Disease-specific survival in the entire group of chondrosarcoma of the femur was 69% (CI95% 63-76) at 5 years. Five-year disease-specific survival in the fracture group was 49% lower than in the control group 75% (p = 0.0001). Survival of patients with grade 1 chondrosarcoma with fracture was significantly less than those without fracture (p = 0.02) but there was no difference in those with grade 2-3 (p = 0.49) and dedifferentiated tumours (p = 0.09). The local recurrence rate of the entire series was 27%. Only dedifferentiated chondrosarcomas with an associated pathological fracture had a significantly higher rate of local recurrence. There was no relationship between development of metastases and fracture. CONCLUSION: A pathological fracture of the femur has a negative prognostic influence in grade 1 chondrosarcoma and increases the risk of local recurrence in dedifferentiated femur chondrosarcomas.

Aneurysmal bone cysts: do simple treatments work?

BACKGROUND: Primary aneurysmal bone cysts (ABCs) are benign, expansile bone lesions commonly treated with aggressive curettage with or without adjuvants such as cryotherapy, methacrylate cement, or phenol. It has been reported that occasionally these lesions heal spontaneously or after a pathologic fracture, and we observed that some ABCs treated at our center healed after biopsy alone. Because of this, we introduced a novel biopsy technique we call "curopsy," which is a percutaneous limited curettage at the time of biopsy, obtaining the lining membrane from various quadrants of the cyst leading to consolidation (curopsy = biopsy with intention to cure). QUESTIONS/PURPOSES: We asked whether (1) a curopsy results in comparable likelihood of healing of the ABC compared with more aggressive approaches involving curettage, (2) the two approaches differ in terms of the likelihood of recurrence after treatment, and (3) the two approaches differ in terms of complications after surgery. METHODS: Between January 1, 1999 and June 30, 2012, 221 patients with a diagnosis of primary ABC were registered in our oncology database. Patients presenting with a pathologic fracture and those seeking a second opinion were excluded. One hundred ninety patients were included in the study. One hundred two (54%) were treated with curopsy and 88 (46%) were treated with curettage after a core needle biopsy. Complete followups were available for 88% (90 of 102) and 93% (80 of 88) of patients in those groups, respectively. During that period, a curopsy was performed for all patients with benign bone lesions with imaging suggestive of classic primary ABCs and for whom the core needle biopsy simply showed blood with no solid component. Curettage after a core needle biopsy was reserved for histologically confirmed primary ABCs, lesions with impending fractures, large lesions, if the ABC was thought to be a secondary disorder, and patients for whom the curopsy failed. All patients were followed up until consolidation of the lesion (mean, 9.6 weeks, range, 3-25 weeks, 95% CI, 8.32-10.9 for curopsy; mean, 11.4 weeks, range, 8-32 weeks, 95% CI, 10.6-12.3 for curettage). The median followup for all patients was 14 months (range, 6-36 months). RESULTS: Of the 102 patients who had curopsy and observation, 83 (81%) required no additional treatment and the lesion resolved. Of the 88 patients who underwent curettage (with or without adjuvant therapy) after core needle biopsy, the success rate was 90% (79 of 88). Local recurrences in both groups (curopsy or curettage) were treated successfully with additional curettage in all but one case. Curopsy in comparison to curettage provided a mean shorter healing time (9.6 versus 11.4, p = 0.01) but there was a higher local recurrence and need for additional intervention rate (18.6% versus 10.2%, p = 0.04). There were no differences in the complications between the treatment groups. CONCLUSIONS: A curopsy is a novel biopsy technique that was successful in resolving ABCs in 81% of the patients in our study. Curopsy, as a biopsy technique, for ABCs needs consideration as it potentially minimizes the number of patients needing a second procedure (a core needle biopsy being the first) as is the current practice. Furthermore, it does not disadvantage the patient or surgeon should additional intervention be needed in the form of curettage with or without adjuvants. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Chondrosarcoma of the Femur: Is Local Recurrence Influenced by the Presence of an Extraosseous Component?

BACKGROUND: Chondrosarcoma (CS) is the second most common surgically treated primary malignancy of the bone. The current study explored the effect of the margin and extraosseous tumor component in CS in the femur on local recurrence (LR), LR-free survival (LRFS), and disease-specific survival (DSS). METHODS: Among 202 patients, 115 were in the proximal extremity of the femur, 4 in the corpus of the femur, and 83 in the distal extremity of femur; 105 patients had an extraosseous tumor component. RESULTS: In the Kaplan-Meier analysis, factors significant for decreased LRFS were the extraosseous tumor component (p < 0.001), extraosseous tumor component arising from the superior aspect (p < 0.001), histological grade (p = 0.031), and narrow surgical margin < 3 mm (p < 0.001). Factors significantly affecting DSS were the histological grade (p < 0.001), extraosseous component (p < 0.001), LR (p < 0.001), metastases (p < 0.001), and surgical margin (p < 0.001). CONCLUSIONS: In CS of the femur, the presence of an extraosseous tumor component has a predictive role in LRFS, and extraosseous tumor component arising from the superior aspect was significant for decreased LRFS. Wide margins were more commonly achieved when the tumor had only an intraosseous component, and the rate of LR was significantly higher in cases with an extraosseous tumor component. When the extraosseous component arose from the superior aspect of the femur, LR occurred more frequently despite achieving adequate margins.

Hoffa's fat pad tumours: what do we know about them?

PURPOSE: We report on a group of patients with tumours in the Hoffa's fat pad (HFP), their clinical presentation, histological type and treatment, including two synovial sarcomas with their clinical follow-up, which have not been described previously in the literature. METHODS: We performed a retrospective review of our prospectively collected database of 25 cases of HFP tumours with at least six months follow-up. RESULTS: The gender, age at presentation (over and under 16 years of age), clinical features, history of trauma, treatment chosen, and complications were recorded. The mean age of the patients was 32 years (three to 47). Six patients were under 16 years old. Pain was the most common symptom, present in 92% (n = 23/25). The final diagnoses included 23 (92%) benign tumours and two (8%) malignant tumours. The most common benign tumour was pigmented villonodular synovitis (PVNS) (48% n = 12). The two malignant tumours were synovial sarcomas and both presented in patients under 16 years old. CONCLUSIONS: Hoffa's fat pad tumours are an uncommon and rarely diagnosed group of lesions that can be misinterpreted as any knee pathology. Although the majority of HFP tumours are benign, malignant tumours should be considered in the differential diagnosis for the paediatric population.

Aseptic failure: how does the Compress(®) implant compare to cemented stems?

BACKGROUND: Failure of endoprosthetic reconstruction with conventional stems due to aseptic loosening remains a challenge for maintenance of limb integrity and function. The Compress(®) implant (Biomet Inc, Warsaw, IN, USA) attempts to avoid aseptic failure by means of a unique technologic innovation. Though the existing literature suggests survivorship of Compress(®) and stemmed implants is similar in the short term, studies are limited by population size and followup duration. QUESTIONS/PURPOSES: We therefore compared (1) the rate of aseptic failure between Compress(®) and cemented intramedullary stems and (2) evaluated the overall intermediate-term implant survivorship. METHODS: We reviewed 26 patients with Compress(®) implants and 26 matched patients with cemented intramedullary stems. The patients were operated on over a 3-year period. Analysis focused on factors related to implant survival, including age, sex, diagnosis, infection, aseptic loosening, local recurrence, and fracture. Minimum followup was 0.32 years (average, 6.2 years; range, 0.32-9.2 years). RESULTS: Aseptic failure occurred in one (3.8%) patient with a Compress(®) implant and three (11.5%) patients with cemented intramedullary stems. The 5-year implant survival rate was 83.5% in the Compress(®) group and 66.6% in the cemented intramedullary stem group. CONCLUSIONS: The Compress(®) implant continues to be a reliable option for distal femoral limb salvage surgery. Data regarding aseptic failure is encouraging, with equivalent survivorship against cemented endoprosthetic replacement at intermediate-term followup. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Is bone scintigraphy necessary in the initial surgical staging of chondrosarcoma of bone?

OBJECTIVE: To assess the value of whole-body bone scintigraphy in the initial surgical staging of chondrosarcoma of bone. METHODS: A retrospective review was conducted of the bone scintigraphy reports of a large series of patients with peripheral or central chondrosarcoma of bone treated in a specialist orthopaedic oncology unit over a 13-year period. Abnormal findings were correlated against other imaging, histological grade and the impact on surgical staging. RESULTS: A total of 195 chondrosarcomas were identified in 188 patients. In 120 (63.8%) patients the reports of bone scintigraphy noted increased activity at the site of one or more chondrosarcomas. In one patient the tumour was outside the field-of-view of the scan, and in the remaining 67 (35.6%) cases, there was increased activity at the site of the chondrosarcoma and further abnormal activity in other areas of the skeleton. Causes of these additional areas of activity included degenerative joint disease, Paget's disease and in one case a previously undiagnosed melanoma metastasis. No cases of skeletal metastases from the chondrosarcoma were found in this series. Multifocal chondrosarcomas were identified in three cases. In two it was considered that all the tumours would have been adequately revealed on the initial MR imaging staging studies. In only the third multifocal case was an unsuspected, further presumed low-grade, central chondrosarcoma identified in the opposite asymptomatic femur. Although this case revealed an unexpected finding the impact on surgical staging was limited as it was decided to employ a watch-and-wait policy for this tumour. CONCLUSION: There is little role for the routine use of whole-body bone scintigraphy in the initial surgical staging in patients with chondrosarcoma of bone irrespective of the histological grade.

Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients.

BACKGROUND: Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases. METHOD: A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society. RESULTS: Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l). CONCLUSIONS: Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.

Patient-oriented functional results of total femoral endoprosthetic reconstruction following oncologic resection.

BACKGROUND AND OBJECTIVES: Functional outcomes following oncologic total femoral endoprosthetic reconstruction (TFR) are lacking. We compared patient-oriented functional results of TFRs to proximal femur and distal femur reconstructions (PFR and DFR). We also compared function and complications with regard to knee and hip componentry. METHODS: Fifty-four TFR patients were identified from three institutional prospective databases. Forty-one had fixed- and 13 had rotating-hinge knees, 37 hemiarthroplasty and 17 total hip arthroplasty componentry. Toronto Extremity Salvage Scores (TESS) for n = 27 were compared between groups and to cohorts of PFR (n = 31) and DFR (n = 85) patients using the Mann-Whitney U-test. RESULTS: Follow-up averaged 4 years. Mechanical complications included five hip dislocations and one femoral malrotation. Four dislocations were in fixed-hinge implants, all in those lacking abductor reattachment. TESS averaged 69.3 ± 17.8, statistically decreased from DFR (P = 0.002) and PFR patients (P = 0.036). No significant differences were detected between patients in the fixed-hinge (n = 18) and rotating-hinge (n = 9) groups (P = 0.944), or total hip (n = 8) and hemiarthroplasty (n = 19) groups (P = 0.633). CONCLUSIONS: TFR is reserved for extreme cases of limb salvage, portending a poor prognosis overall. Function reflects additive impairments from PFR and DFR. TFR outcomes differ little with rotating- or fixed-hinge, total hip or hemiarthroplasty implants.

Stewart-Treves syndrome and the use of positron emission tomographic scanning.

In this article, we provide an account of two rare cases of Stewart-Treves syndrome, that is, cutaneous angiosarcoma secondary to lymphedema, treated at our center. Unusually, both occurred in the lower extremity. The first case was treated initially with a wide local excision, followed by a further re-excision, and eventually an above-the-knee amputation because of recurrence. In the second case, a hindquarter amputation was undertaken after a positron emission tomographic scan, which revealed the extent and spread of the lesions. In cases of cutaneous angiosarcoma, a positron emission tomographic scan can be extremely helpful in demonstrating the extent of subcutaneous spread and planning surgical management.

Regional variation in the survival of patients with a soft-tissue sarcoma of the extremity and trunk wall under a centralized care system : what has been the impact of national policies in the UK?

AIMS: While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. METHODS: The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management. RESULTS: There had been a significant difference in survival between patients referred from the North East, North West, East Midlands, West Midlands, South West, and Wales in the pre-NICE era (five-year disease-specific survival (DSS); South West, 74% vs North East, 47% (p = 0.045) or West Midlands, 54% (p = 0.049)), which was most evident for patients with a high-grade STS. However, this variation disappeared in the post-NICE era, in which the overall DSS for high-grade STS improved from 47% to 68% at five years (p < 0.001). Variation in the size of the tumour closely correlated with the variation in DSS, and the overall size of the tumour and incidence of metastasis at the time of diagnosis also decreased after the national policies were issued. CONCLUSION: The survival of patients with a STS improved and regional variation corrected after the introduction of national policies, as a result of a decreasing size of tumour and incidence of metastasis at the time of diagnosis, particularly in patients with a high-grade STS. This highlights the positive impact of national guidelines on regional variation in the presentation, management, and outcome in patients with a STS. Cite this article: Bone Joint J 2021;103-B(9):1541-1549.

Limb-salvage reconstruction following resection of pelvic bone sarcomas involving the acetabulum.

AIMS: Limb salvage for pelvic sarcomas involving the acetabulum is a major surgical challenge. There remains no consensus about what is the optimum type of reconstruction after resection of the tumour. The aim of this study was to evaluate the surgical outcomes in these patients according to the methods of periacetabular reconstruction. METHODS: The study involved a consecutive series of 122 patients with a periacetabular bone sarcoma who underwent limb-salvage surgery involving a custom-made prosthesis in 65 (53%), an ice-cream cone prosthesis in 21 (17%), an extracorporeal irradiated autograft in 18 (15%), and nonskeletal reconstruction in 18 (15%). RESULTS: The rates of major complications necessitating further surgery were 62%, 24%, 56%, and 17% for custom-made prostheses, ice-cream cone prostheses, irradiated autografts and nonskeletal reconstructions, respectively (p = 0.001). The ten-year cumulative incidence of failure of the reconstruction was 19%, 9%, 33%, and 0%, respectively. The major cause of failure was deep infection (11%), followed by local recurrence (6%). The mean functional Musculoskeletal Tumour Society (MSTS) scores were 59%, 74%, 64%, and 72%, respectively. The scores were significantly lower in patients with major complications than in those without complications (mean 52% (SD 20%) vs 74% (SD 19%); p < 0.001). For periacetabular resections involving the ilium, the mean score was the highest with custom-made prostheses (82% (SD 10%)) in patients without any major complication; however, nonskeletal reconstruction resulted in the highest mean scores (78% (SD 12%)) in patients who had major complications. For periacetabular resections not involving the ilium, significantly higher mean scores were obtained with ice-cream cone prostheses (79% (SD 17%); p = 0.031). CONCLUSION: Functional outcome following periacetabular reconstruction is closely associated with the occurrence of complications requiring further surgery. For tumours treated with periacetabular and iliac resection, skeletal reconstruction may result in the best outcomes in the absence of complications, whereas nonskeletal reconstruction is a reasonable option if the risk of complications is high. For tumours requiring periacetabular resection without the ilium, reconstruction using an ice-cream cone prosthesis supported by antibiotic-laden cement is a reliable option. Cite this article: Bone Joint J 2021;103-B(4):795-803.

Impact of NICE guidelines on the survival of patients with soft-tissue sarcomas.

AIMS: Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. METHODS: A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated. RESULTS: The mean size of the tumour was significantly smaller at the time of diagnosis (10.3 cm (SD 6.5) vs 9.1 cm (SD 6.2); p < 0.001) and the number of patients who had undergone an inadvertent excision significantly decreased (28% (n = 389) vs 20% (n = 204); p < 0.001) following the introduction of the NICE guidelines. The five-year DSS was 63% in the pre-NICE and 71% in post-NICE groups (p < 0.001). The improved survival was more significant for those with a high-grade tumour (pre-NICE, 48%; post-NICE, 68%; p < 0.001). In those with a high-grade tumour, the mean size of the tumour (11.6 cm (SD 6.2) vs 9.6 cm (SD 5.8); p < 0.001) and the number of patients with metastasis at the time of diagnosis (15% (n = 124 vs 10% (n = 80); p = 0.007) significantly decreased in the post-NICE group. CONCLUSION: An improvement in survival was seen after the introduction of the NICE guidelines, especially in patients with a high-grade STS. More patients were referred at an earlier stage, indicating a clearer pathway after the issue of national policy for the management of STSs in the UK. Cite this article: Bone Joint J 2021;103-B(3):569-577.